Frontal temporal dementia.

Frontal temporal dementia (also known as frontotemporal dementia, FTD) is a rare neurodegenerative brain disease characterized by deterioration of cognitive and behavioral functions. It is called frontotemporal dementia because it initially affects the frontal and temporal lobes of the brain.

The main features of frontal temporal dementia include:

1. Changes in behavior and personality: Patients may exhibit impaired social behavior, become less inhibited, immoral, or eccentric. Problems with emotion and affect management may occur.
2. Cognitive decline: In the initial stages of FTD, patients may retain relatively normal intellectual abilities, but over time, problems with language (apraxia of speech) and tasks related to planning and decision-making may occur.
3. Social disinhibition: Patients with FTD may exhibit maladaptive behavior in social situations and a loss of capacity for social norms.
4. Gradual worsening: Over time, the symptoms of frontal temporal dementia worsen and patients become increasingly dependent on care.

Frontal temporal dementia has several subtypes, each of which can present with varying degrees of symptoms and impairment. As yet, there is no specific treatment that can slow the progression of FTD, and the approach to care is largely limited to symptom management and support for the patient and family. [1]

This is an important condition and consultation with an experienced neurologist or neurodegenerative disease specialist is necessary for accurate diagnosis and management of frontal temporal dementia.

Causes of the frontal temporal dementia.

Frontal temporal dementia has many different causes, and research in this area is ongoing. Mainly, FTD is a neurodegenerative disease, meaning that it involves damage and death of neurons in certain areas of the brain. Causes of FTD include:

1. Genetic factors: Genetic mutations are considered one of the main causes of FTD. Some familial forms of FTD are associated with mutations in genes such as C9orf72, GRN (preapolypeptide derived protein), MAPT (gene for tauprotin) and others. People with related forms of FTD have a higher risk of getting the disease. [2]
2. Protein aggregation: It is possible that FTD is associated with the accumulation of abnormal protein structures such as tauprotein that form neuronal inclusions and cause neuronal damage.
3. Neuroinflammation: Brain inflammation and neuroinflammation may also be associated with the development of FTD.
4. Other Factors: Research is ongoing to better understand other possible factors that may contribute to FTD, such as the environment and environmental factors.

Symptoms of the frontal temporal dementia.

Some of the main symptoms of frontotemporal dementia include:

1. Changes in behavior and personality: Patients may exhibit abnormal or inappropriate behavior such as apathy, immorality, unwillingness to follow social norms, inconsiderateness, or impaired personal hygiene.
2. Emotional disorders: Changes in emotional stability may occur, as well as impairments in the ability to understand and express emotions. Patients may become emotionally detached or may exhibit excessive emotion.
3. Cognitive decline: Although FTD primarily affects behavior and emotions, over time it can also lead to impairments in memory, language, and other cognitive functions. This can manifest itself in difficulties with expressive and receptive language, as well as impaired decision-making and problem-solving abilities.
4. Decreased social adjustment: Patients may experience difficulties in interpersonal relationships and social adjustment. They may exhibit antisocial behavior, inability to understand the feelings of others and to maintain social connections.
5. Decreased self-control: Patients may have difficulty with self-control and control over their actions. This can lead to compulsive or impulsive behaviors. [3]

Stages

Frontal temporal dementia progresses through several stages, which are characterized by progression of symptoms and deterioration of cognitive and behavioral functions. The stages of FTD may vary depending on the research system and specific clinical cases, but three main stages are usually distinguished:

1. Early stage (mild):

   • In this stage, the patient may exhibit mild and unobtrusive symptoms that can be easily underestimated or mistakenly attributed to stress or depression.
   • Characteristic symptoms include changes in behavior and personality, as well as mild apathy and loss of interest in usual activities.
   • Cognitive functions such as memory and orienting may remain relatively preserved.

2. Middle stage (moderate):

   • In this stage, FTD symptoms become more pronounced and affect the patient's daily life.
   • The patient may experience severe behavioral changes including aggression, apathy, maladaptive social behavior, and compulsivity.
   • Cognitive function begins to deteriorate, which can lead to problems with memory, thinking, and speech.
   • Patients may also experience problems with orientation in space and time.

3. Late stage (severe):

   • In the last stage of FTD, patients may be completely dependent on care and assistance.
   • The symptoms associated with cognitive deterioration become very pronounced, and patients may completely lose the ability to live independently.
   • Physical problems, such as swallowing and movement problems, may also become more serious.

Forms

Frontotemporal dementia includes several forms that differ in terms of dominant symptoms and brain changes. The main forms of FTD include:

1. Behavioral variant frontotemporal dementia (bvFTD): This form is characterized by marked changes in behavior, personality, and social adaptation. Patients may exhibit immoral, disinhibited, compulsive, or apathetic behavior. Cognitive functions such as memory and language may be preserved in the initial stages.
2. Aphasic form (primary progressive aphasia, PPA): This form of FTD affects language functions. There are several subtypes of PPA, including semantic degradation aphasia (svPPA), non-fluent/asymmetric primary aphasic disorder aphasia (nfvPPA), and aphasia associated with primary projective aphasia (PPAOS). Symptoms include impaired ability to understand and use words, as well as impairments in articulation.
3. Alzheimer's disease-like FTD: This form of FTD shows symptoms similar to Alzheimer'sdisease, including memory loss and cognitive impairment. Unlike Alzheimer's disease, however, FTD usually preserves learning and spatial orientation abilities.
4. Corticobasal degeneration (CBD): This form of FTD presents with symptoms including atypical movement disorders such as hyperkinesis and muscle rigidity. Cognitive function is also impaired.
5. Progressive supranuclear palsy (PSP): This form of FTD is characterized by impaired motor coordination, decreased ability to hold the head and gaze, and cognitive impairment. [4]

Diagnostics of the frontal temporal dementia.

Diagnosing frontal temporal dementia is a complex process that involves several steps and methods to identify this neurodegenerative disease. Here are the common steps and methods for diagnosing FTD:

1. Clinical examination and history:

   • The doctor conducts a detailed examination of the patient and collects a medical history to assess the symptoms and the duration of their presence. Particular attention is paid to changes in the patient's mental and cognitive state.

2. Psychological tests and assessment of cognitive function:

   • The patient may be offered a variety of psychological tests and cognitive assessments to help identify impairments in memory, thinking, speech, and behavior.

3. Magnetic resonance imaging (MRI):

   • Brain MRI can be used to visualize structural changes in the brain, such as decreased brain volume and atrophy of the frontal and temporal lobes, which are characteristic of FTD.

4. Positron emission tomography (PET):

   • PET can be performed to study metabolic changes in the brain, including changes in glucose activity and protein aggregates associated with FTD.

5. Brain Spectrum Survey:

   • This study can be performed to detect the presence of biochemical markers such as amyloid and tau proteins that may be associated with FTD.

6. Exclusion of other causes:

   • It is important to rule out other possible causes of cognitive and mental impairment, such as Alzheimer's disease and psychiatric disorders that may mimic the symptoms of FTD.

7. Consultation with a neurologist and neuropsychologist:

   • Consultation with specialists in neurology and neuropsychology can assist in evaluating and interpreting the results of diagnostic tests.

Differential diagnosis

Differential diagnosis of frontal temporal dementia is important to distinguish this neurodegenerative disease from other types of dementia and neurologic conditions. Below are some basic steps and factors that can help the clinician make a differential diagnosis of FTD:

1. Symptom assessment: The clinician should conduct a detailed examination of the patient and examine the characteristics of the symptoms. Symptoms of FTD may include behavioral changes, social disinhibition, immoral behavior, impaired planning and decision-making abilities, and aphasia (speech impairment). It is important to determine which symptoms are predominant.
2. Clinical examinations: The physician may perform a variety of clinical tests and assessments to measure the patient's cognitive function, emotional state, and behavior.
3. Neuroimaging: Brain imaging with techniques such as magnetic resonance imaging (MRI) and positron emission tomography (PET) can help visualize brain structure and function, as well as identify abnormalities that may be associated with FTD.
4. Genetic testing: In cases with a family history of FTD or other neurodegenerative diseases, genetic testing may be useful to detect the presence of specific mutations associated with FTD.
5. Ruling out other causes: The doctor should rule out other possible causes of dementia such as Alzheimer's disease, [5] Parkinson's disease, vascular dementia, and other neurological and psychiatric conditions.
6. Psychological and social assessment: Assessment of psychological and social functioning can be useful in identifying changes in behavior and the impact of FTD on the patient and family.
7. Consultation of specialists: In some cases, it may be necessary to consult neuropsychologists, neurologists, psychiatrists and other specialists to clarify the diagnosis.

Treatment of the frontal temporal dementia.

It is a progressive disease for which there is no specific treatment method, but some approaches can be used to manage symptoms and maximize patient comfort:

1. Medications: Many medications used to treat Alzheimer's disease and other neurodegenerative diseases can be used to manage the symptoms of frontal temporal dementia. These medications may include cholinesterase inhibitors and memantine.
2. Psychological support: Patients and their families often need psychological and emotional support. Psychologists and psychiatrists can help to cope with the emotional difficulties associated with the disease and develop strategies for managing behavioral changes.
3. Speechand physical therapy: Speech therapy can help patients with maintaining or improving communication skills. Physical therapy and exercise can help maintain physical mobility and reduce the risk of side effects of a sedentary lifestyle.
4. Special diet and nutrition: In some cases, a special high-protein, low-carbohydrate diet is recommended for patients with frontal temporal dementia.
5. Symptom management and safety: Because patients with frontal temporal dementia may exhibit bizarre or aggressive behavior, it is important to ensure their safety and the safety of those around them. This may include controlling access to dangerous objects and ensuring proper supervision.
6. Clinical trials: In some cases, patients are encouraged to participate in clinical trials that investigate new treatments and medications for frontal temporal dementia. [6]

Forecast

The prognosis for frontotemporal dementia can be variable depending on several factors, including the form of FTD, the patient's age, the degree of disease progression, and the presence of additional diseases. The overall prognosis for FTD is usually poor because it is a progressive neurodegenerative disease.

Here are some of the key aspects of the FTD forecast:

1. Time of symptom onset: Prognosis may depend on how early a diagnosis is made and appropriate therapy started. Seeing a doctor early and starting treatment can help slow the progression of the disease.
2. Form of FTD: As mentioned earlier, there are several forms of FTD, and prognosis may vary depending on the form. For example, the frontal form of FTD, characterized by behavioral changes, may have a poorer prognosis compared to the aphasic form, which is dominated by language symptoms.
3. Individual factors: The age of onset of symptoms, the patient's general health, and the presence of other medical conditions can also affect prognosis.
4. Family support and care: The quality of care and support from family and caregivers can have a significant impact on a patient's quality and length of life.
5. Complications and associated problems: FTD can lead to various complications such as infections, pneumonia, etc., which can also affect the prognosis.

The overall prognosis of FTD is usually poor, and the disease progresses over time, leading to behavioral disturbances, cognitive impairment, and loss of independence.

Life expectancy

Life expectancy in frontotemporal dementia can vary greatly depending on many factors, including the form of FTD, age of onset of symptoms, degree of disease progression, and individual patient characteristics.

FTD usually begins in middle age, often before age 65, which distinguishes it from the more common Alzheimer's disease. Life expectancy after diagnosis of FTD can range from a few years to decades, but the average life expectancy after diagnosis is usually about 7-8 years.

However, it is worth noting that FTD is a progressive neurodegenerative disease and symptoms worsen over time. When the disease reaches a more advanced stage, it can lead to complete dependence on care and complications such as infections or pneumonia, which can shorten life expectancy.

The life expectancy and prognosis of FTD also depends on the individualized support and care the patient receives and how successfully symptoms and complications are managed. Early referral, assessment and support from a social worker, family support and the use of appropriate therapeutic and supportive techniques can help to improve the patient's quality of life and prolong its duration.

List of authoritative books and studies related to the study of frontal temporal dementia

1. "Frontotemporal Dementia: Syndromes, Imaging, and Molecular Characteristics" - authors: Giovanni B. Frisoni, Philip Scheltens (Year: 2015)
2. "Frontotemporal Dementia: Neurological Disease and Therapy" - by David Neary, John R. Hodges (Year: 2005)
3. "Frontotemporal Dementia: From Bench to Bedside" - by Bruce L. Miller (Year: 2009)
4. "Frontotemporal Dementia Syndromes" - by Mario F. Mendez (Year: 2021)
5. "Frontotemporal Dementia: Clinical Phenotypes, Pathophysiology, Imaging Features, and Treatment" - by Erik D. Roberson (Year: 2019)
6. "Frontotemporal Dementia: Causes, Symptoms, Diagnosis, Treatment and Care" - by George W. Smith (Year: 2019)
7. "Frontotemporal Dementia: Advances in Neuroimaging and Neuropathology" - by Giovanni B. Frisoni (Year: 2018)
8. "Frontotemporal Dementia: Syndromes, Genetic Analysis, and Clinical Management" - by Elisabet Englund (Year: 2007)
9. "Behavioral Neurology and Neuropsychiatry" - by David B. Arciniegas (Year: 2013)

Literature

Gusev, E. I. Neurology : national guide : in 2 vol. / ed. By E. I. Gusev, A. N. Konovalov, V. I. Skvortsova. - 2nd ed. Moscow : GEOTAR-Media, 2021. - Т. 2.